Case History #34

Date of Consultation: 1-11-1980

HISTORY OF PRESENT ILLNESS:

This 40-year-old right-handed white female was referred for neurological consultation for evaluation of involuntary movements.  The patient was unable to offer a coherent history of any neurologic problems, and, in fact, she seemed to deny that any neurologic difficulties were present whatsoever.  The patient was accompanied by a social worker who indicated that she had noticed that the patient was having difficulty walking for the past two years.  This evidently began gradually and was a steadily progressive problem.  The social worker also described slurred speech and abnormal arm movements over the previous two years that seemed to be slowly worsening.  The patient was evaluated in 1976 by a neurologist who found no evidence of neurologic disease except for the possibility of a "mild dementia". 

FAMILY HISTORY:

The patient and the social worker indicated that there was a strong family history of some sort of neurologic disease.  This problem was present in the patient's mother, grandmother, and one of her aunts.  The patient was unable to describe the symptoms of the disease clearly but mentioned that her mother "jerked a lot and didn't talk much".  The patient's mother died in her 40s or 50s. 

NEUROLOGICAL EXAMINATION:

Mental Status Exam:  The patient was oriented to person, place, year, and month, but was unable to recall the day of the month.  Her fund of general information was only fair.  She performed calculations quite poorly.  Her long term and short term memory functions were both judged to be only fair.  The social worker indicated that she had observed the patient talking to herself and also indicated that at times the patient became quite paranoid.  There was also an indication that the patient had experienced both auditory and visual hallucinations. 

Cranial Nerves:  Intact.

Motor System:  There were no areas of focal muscular atrophy.  There were no fasciculations.  Muscle tone and power were normal in all 4 limbs.  The patient exhibited a moderate to marked amount of spontaneous involuntary movements.  These involved both proximal and distal portions of both upper limbs.  The movements frequently propelled her arms into unusual postures.  There were both rapid, irregular, jerky movements and slower, writhing and twisting movements.  However, she was quite adept at masking these movements with other accessory movements.  She also tended to "sit on her hands".  The patient also exhibited small amplitude twitching and twisting movements of the mouth and face that occurred spontaneously and irregularly.  The tongue and lips often were protruded and somewhat contorted.  The patient spoke with a significant dysarthria. 

Reflexes:  Deep tendon reflexes were slightly brisk but equal bilaterally.  The patient exhibited a positive snout reflex and a brisk jaw jerk.  The plantar reflexes were flexor bilaterally.

Sensory System:  Intact in all 4 limbs.

Cerebellar Function:  Intact. 

Gait and Stance:  Regular gait was performed on a slightly widened base and was somewhat slow.  At times, there were abrupt lurches to either side, but the patient did not lose her balance or stagger.  The Romberg test was negative.

Questions:

1.  How would you describe or classify this patient’s involuntary movements?
    
2.  Indicate the level(s) of the neurologic lesion in this case. 

3.  Indicate the structures involved by the pathologic process. 

4.  The findings in this case represent a classic neurologic syndrome.  What is the name of this syndrome? 

5.  In general, what type of pathologic process is involved in this case? 

6.  What diagnostic procedure(s) would you undertake at this point?